Noriaki Kameda

Malignant glomus tumor: a case report and review of the literature.

This report concerns a malignant glomus tumor, a rare soft tissue tumor that was examined immunohistochemically and ultrastructurally. It occurred in a 44-year-old male patient who had suffered from dull pain and stiffness in the right thigh for 10 months. Radiographic examination revealed a well-defined osteolytic lesion in the diaphysis of the right femur. Hypervascularity of the tumor was observed angiographically. Computed tomographic and magnetic resonance examinations showed an intramuscular mass invading the marrow space of the femur. Wide resection was performed after open biopsy. Histologically, round to polygonal tumor cells revealed a uniform appearance of round to ovoid nuclei with single large nucleoli and slightly eosinophilic cytoplasm, forming solid sheets of cells interrupted by vessels of varying size. A few mitotic figures and vascular invasion were observed. Immunohistochemically, vimentin and alpha-smooth muscle actin were stained intensely, and muscle actin was positive for tumor cells of the perivascular area. Tumor cells were negative for desmin, factor VIII-related antigen, S-100 protein, neurofilament, cytokeratin, and epithelial membrane antigen. Ultrastructurally, tumor cells were characterized by many cytoplasmic processes, pinocytotic vesicles, plasmalemmal dense plaques, and scattered microfilaments in the cytoplasm. Few cell junctions and focal basement membrane-like structures were observed. No recurrence or metastasis was noted 57 months after operation. This case was considered to be a malignant glomus tumor, that is, a glomangiosarcoma arising de novo.

Detection of Vulnerable Coronary Plaques by Color Fluorescent Angioscopy

OBJECTIVES This study was carried out to detect vulnerable coronary plaques by color fluorescent angioscopy. BACKGROUND Collagen fibers (CFs) mainly provide mechanical support to coronary plaques. Oxidized low-density lipoprotein (Ox-LDL) induces macrophage proliferation, which in turn destroy CFs while accumulating lipids. As such, demonstration of the absence of CFs, deposition of lipids, and the Ox-LDL may suggest plaque instability. METHODS Fluorescence of the major components of the atherosclerotic plaques was examined by fluorescent microscopy using a 345-nm band-pass filter and 420-nm band-absorption filter (A-imaging). Fluorescence of Ox-LDL was examined using a 470-nm band-pass filter and 515-nm band-absorption filter (B-imaging) and Evans blue dye as an indicator. Fluorescence in 57 excised human coronary plaques was examined by A-imaging color fluorescent angioscopy. Oxidized LDL in 31 excised coronary plaques and in 12 plaques of 7 patients was investigated by B-imaging color fluorescent angioscopy. RESULTS Collagen I, collagen IV, and calcium exhibited blue, light blue, and white autofluorescence, respectively. In the presence of beta-carotene which coexists with lipids in the vascular wall, collagen I and IV exhibited green, collagen III and V white, cholesterol yellow, cholesteryl esters orange fluorescence. Oxidized LDL exhibited reddish brown fluorescence in the presence of Evans blue dye. Therefore, coronary plaques exhibited blue, green, white-to-light blue, or yellow-to-orange fluorescence based on plaque composition. Histological examination revealed abundant CFs without lipids in blue plaques; CFs and lipids in green plaques; meager CFs and abundant lipids in white-to-light blue plaques; and the absence of CFs and deposition of lipids, calcium, and macrophage foam cells in the thin fibrous cap in yellow-to-orange plaques, indicating that the yellow-to-orange plaques were most vulnerable. Reddish brown fluorescence characteristic of Ox-LDL was observed in excised coronary plaques, as also in patients. CONCLUSIONS Color fluorescent angioscopy provides objective information related to coronary plaque composition and may help identify unstable plaques.

A case of ectopic breast cancer with a literature review

A 63-year-old woman was referred to our hospital because of a right axillary nodule in 2004. Physical examination showed a spherical nodule measuring 0.5cm in diameter in the right axilla. No mass was palpable in either breast. Mammograms were normal. Ultrasonography revealed a subcutaneous hypoechoic mass 0.7mm in maximum diameter in the right axilla. The patient underwent an excisional biopsy. Histological examination revealed an invasive ductal carcinoma (scirrhous carcinoma) in ectopic breast tissue. The patient subsequently underwent a wide local excision of the tissue surrounding the biopsy scar, with axillary lymph node dissection. Histologically, no residual tumor or nodal metastasis was found. Postoperatively, she received endocrine therapy and remains well, without any evidence of recurrence 4 years 10 months after operation. Cancer of the ectopic breast tissue is rare, and most cases present as a solitary axillary mass. Long-term outcomes remain unclear. We present a case of breast carcinoma in the axillary ectopic mammary gland and summarize the clinical features of 94 cases, including ours, in Japan. We also compare long-term survival between ectopic breast cancer and usual breast cancer according to TNM T stage and lymph node metastasis.

Primary carcinoid tumor of the testis: Immunohistochemical, ultrastructural and FISH analysis with review of the literature

Primary carcinoid tumor of the testis only accounts for 0.2% of all carcinoid tumors in Japan. The clinicopathological features, including differences between Japanese and Europe/United States (Eur/US) series’, which are known in ovarian carcinoids, have not yet been sufficiently recognized. In the present study, five testicular carcinoids from various clinicopathological viewpoints were analyzed, with a review of the literature. All 15 cases in Japan, including 10 previously reported, were insular carcinoids. Three of the 15 cases (20%) were associated with a teratoma. Serotonin production was confirmed in at least 10 cases (five cases in the present study and five cases in previous studies), but carcinoid syndrome was present in only two cases. Three of the 15 cases (20%) showed distant metastases, and the tumors that metastasized were larger than those that did not. These features were almost similar to those observed in the Eur/US series. Three pure carcinoids were also studied for the number of sex chromosomes by fluorescence in situ hybridization (FISH). They did not show a significant numerical abnormality of the X chromosome, which is common in testicular germ cell tumors. Therefore, the genetic background of pure carcinoids might be different from that of common germ cell tumors.

Peripheral primitive neuroectodermal tumor of the jugular foramen: Case report

OBJECTIVE AND IMPORTANCE Peripheral primitive neuroectodermal tumor (pPNET) is a rare type of tumor, most commonly found in the limbs of children and young adults. The authors report an extremely rare case of pPNET located at the jugular foramen whose clinical course demonstrated rapid progression. CLINICAL PRESENTATION A 23-year-old man presented with a 2-month history of progressive hoarseness and dysphagia. The neuroradiological appearance of the lesion was a jugular foramen tumor. INTERVENTION The patient underwent a partial resection of the tumor through a far lateral suboccipital craniotomy. After surgery, the patient experienced an unexpected deterioration in consciousness. Magnetic resonance images on postoperative Day 18 revealed rapid and large expansion of the residual tumor into the posterior fossa. No adjuvant therapy was administered, and the patient died 6 weeks after diagnosis. CONCLUSIONThe pathological diagnosis of the surgical specimen was pPNET, according to the findings of hematoxylin and eosin and immunohistochemical stainings. To the best of our knowledge, this is the first reported case of pPNET at the jugular foramen.

True Malignant Mixed Tumor of the Submandibular Gland

A case of true malignant mixed tumor of the submandibular gland is reported. The submandibular tumor, occurring in a 52‐year‐old man, started to grow rapidly after a long history without any change in size. Surgical resection was carried out and the resected tumor measured 5.5 cm with a cut surface showing mixed solid structures. Microscopically, the tumor had both carcinomatous and sarcomatous elements, the former consisting of poorly differentiated adenocarcinoma with squamous cell differentiation and the latter consisting of osteosarcoma with chondrosarcomatous and fibrosarcomatous elements. A remnant of benign pleomorphic adenoma could also be identified. Immunohistochemical study demonstrated keratin and epithelial membrane antigen in the carcinoma cells and vimentin in all elements of the osteosarcoma. It is assumed from these clinical and histological findings that the tumor had transformed from a pre‐existing benign pleomorphic adenoma. Acta Pathol Jpn 40: 137–142, 1990.

Myxoid angioblastomatosis of bones. A case report of a rare, multifocal entity with light, ultramicroscopic, and immunopathologic correlation.

An example of multicentric, skeletal, myxoid angioblastomas in a Japanese woman is reported. The disease was symptomatic at age 12 years and was characterized by slowly progressive, multiple, lytic bone defects. In addition the patient had juvenile hypertension, and, at age 20 years, had focal brain infarction. The primitive vascular nature of the process was supported by the following observations: occasional erythrocytes within cytoplasmic lumina and capillary-like cellular tubes; Weibel-Palade bodies, numerous pinocytotic vesicles, prominent microvilli, elaborate intercellular contacts, desmosomes, and numerous arrays of fine intracytoplasmic filaments by electron microscopy; and, in addition, Factor VIII positivity. The clinical findings in this case are more consistent with a multicentric, rather than a metastatic process. The name myxoid angioblastomatosis of bones is appropriate.

Designation of enzyme activity of glycine-N-acyltransferase family genes and depression of glycine-N-acyltransferase in human hepatocellular carcinoma

The human glycine-N-acyltransferase (hGLYAT) gene and two related-genes (GLYATL1 and GLYATL2) were isolated. Human GLYAT, GLYATL1, and GLYATL2 cDNAs were isolated and shown to encode polypeptides of 295, 302, and 294 amino acids, respectively. GLYAT catalyzes glycine-N-acyltransfer reaction with benzoyl-CoA acting as a typical aralkyl transferase, while GLYATL1 catalyzed glutamine-N-acyltransfer reaction with phenylacetyl-CoA as an arylacetyl transferase. GLYAT was shown to be expressed specifically in the liver and kidney, and the cellular localization of GLYAT protein was restricted to the mitochondria. Interestingly, labeling using highly affinity purified anti-GLYAT antibody revealed that GLYAT expression was suppressed in all hepatocellular carcinomas, but not in other liver diseases. hGLYAT repression in cancerous cells in the liver was controlled at the transcriptional level. hGLYAT is a good candidate as a novel marker of hepatocellular carcinoma and may be a key molecule in the transition between differentiation and carcinogenesis of liver cells.

Parasellar chondroma: A case report

A case of a 19-year-old man with a right parasellar chondroma examined with computed tomography (CT) and nuclear magnetic resonance (NMR) scan is reported. The literature about CT and NMR scan findings of intracranial chondroma is reviewed. Typical CT findings are a round, high density mass with calcification, that is only slightly enhanced by contrast medium.

Primary leiomyosarcoma of bone. A case report and review of the literature.

A 62‐year‐old female with primary leiomyosarcoma of the left femur is reported with a review of 21 cases reported in the literature. The resected specimen showed that the tumor extended from the femoral head to the diaphysis for 13cm in length. The tumor showed mainly intramedullary proliferation, but extraosseous growth was also noted at the great trochanter. Microscopic examination revealed well differentiated leiomyosarcoma characterized by interlacing bundles of fusiform cells with eosinophilic cytoplasm and rod‐shaped hyperchromatic nuclei. PAP stain of actin on the tumor cells was positive. On electron microscopy, microfilament of 6–8 nm in diameter, dense bodies, plnocytotic vesicles, marginal attachment plate, and basal lamina were noted. The patient died with pulmonary metastasis, 1 year and 7 months after the operation. An autopsy showed metastases in the right pelvic cavity and bilateral lungs, and confirmed the primary site to be the left femur.